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Cardiovascular surgery in patients with congenital plasma coagulopathies
J C Vander Woude1, J D Milam, W E Walker
1Department of Cardiovascular Surgery, Texas Heart Institute, Houston 77225.
Insights
Cardiovascular operations in patients with congenital bleeding disorders are feasible with careful management. Perioperative hematology support and blood component therapy minimize risks for these complex surgical cases.
Area of Science:
- Cardiology
- Hematology
- Surgical Oncology
Background:
- Congenital bleeding disorders pose significant risks for major surgery.
- Patients with hemophilia A, hemophilia B, Factor XI deficiency, von Willebrand's disease, and dysfibrinogenemia require specialized perioperative care.
- Previous literature on cardiovascular surgery in this population is limited.
Purpose of the Study:
- To evaluate the safety and outcomes of cardiovascular operations in patients with known congenital bleeding disorders.
- To identify key management strategies for minimizing morbidity and mortality in this cohort.
Main Methods:
- Retrospective review of 15 cardiovascular operations performed between 1978 and 1986 on 13 patients with documented congenital bleeding disorders.
- Procedures included aortocoronary bypass grafting, cardiac valve surgery, aortic graft placement, and carotid endarterectomy.
- Perioperative management focused on blood component replacement therapy guided by routine coagulation factor level monitoring.
Main Results:
- No deaths occurred among the 13 patients.
- Two patients experienced hemorrhagic complications requiring reexploration.
- Successful cardiovascular procedures were achieved with appropriate hematologic support and management.
Conclusions:
- Cardiovascular operations can be performed safely in patients with congenital coagulation disorders.
- Effective perioperative management, including hematology consultation and blood component therapy, is crucial.
- Careful monitoring of coagulation status is essential for successful outcomes in these high-risk patients.
Abstract:
From 1978 to 1986, fifteen cardiovascular operations were performed on 13 patients with known congenital bleeding disorders. The patients (10 men and 3 women) had a mean age of 51.1 +/- 3.4 years. Four were seen with cardiovascular lesions and documented hemophilia A (Factor VIII deficiency); 3 had hemophilia B (Factor IX deficiency); 3 had Factor XI deficiency; 2 had von Willebrand's disease, and 1 had dysfibrinogenemia. All patients had a history of major hemorrhage after dental extractions or general surgical procedures, and had clearly documented coagulation disorders on hematological evaluation. Elective cardiovascular procedures performed in these patients included aortocoronary bypass grafting (eight), cardiac valve replacement or repair (five), aortic graft placement (one), and carotid endarterectomy (one). The mainstay of perioperative management included appropriate replacement therapy with blood components. Coagulation factor levels were measured routinely to guide therapy. There were no deaths. Two hemorrhagic complications necessitated reexploration. We conclude that in patients known to have congenital coagulation disorders, cardiovascular operations using systemic heparinization can be performed with minimal morbidity and mortality when carried out with preoperative and perioperative support from the hematology service, adequate replacement therapy using blood components, and careful monitoring of the coagulation status.